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pediatric heart failure

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https://www.readbyqxmd.com/read/29783821/-clinical-and-genetic-analysis-of-eleven-pediatric-patients-with-alagille-syndrome
#1
L Guo, S T Zhao, Y Cheng, M Deng, H Li, Y Z Song, X R Cai, Q Zhou
Objective: To explore the clinical and molecular genetic features of patients with Alagille syndrome (AS). Methods: The clinical data of eleven pediatric patients, who were suspected to have AS at the Department of Pediatrics in the First Affiliated Hospital of Jinan University from August 2010 to March 2017, were collected and analyzed. Genomic DNA was extracted from peripheral blood leukocytes of the patients and their parents. For 5 patients collected before March 2006, all JAG1 exons and their flanking sequences were directly sequenced...
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29777282/ambulatory-intravenous-inotropic-support-and-or-levosimendan-in-pediatric-and-congenital-heart-failure-safety-survival-improvement-or-transplantation
#2
Sotiria C Apostolopoulou, George A Vagenakis, Alexandros Tsoutsinos, Felicia Kakava, Spyridon Rammos
End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29770299/regenerative-medicine-therapy-for-single-ventricle-congenital-heart-disease
#3
REVIEW
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
https://www.readbyqxmd.com/read/29766225/potential-common-pathogenic-pathways-for-the-left-ventricular-noncompaction-cardiomyopathy-lvnc
#4
Ying Liu, Hanying Chen, Weinian Shou
Ventricular trabeculation and compaction are two essential morphogenetic events for generating a functionally competent ventricular wall. A significant reduction in trabeculation is usually associated with hypoplastic wall and ventricular compact zone deficiencies, which commonly leads to embryonic heart failure and early embryonic lethality. In contrast, the arrest of ventricular wall compaction (noncompaction) is believed to be causative to the left ventricular noncompaction (LVNC), a genetically heterogeneous disorder and the third most common cardiomyopathy among pediatric patients...
May 15, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29757759/mechanical-circulatory-support-of-the-right-ventricle-for-adult-and-pediatric-patients-with-heart-failure
#5
Steven G Chopski, Nohra M Murad, Carson S Fox, Randy M Stevens, Amy L Throckmorton
The clinical implementation of mechanical circulatory assistance for a significantly dysfunctional or failing left ventricle as a bridge-to-transplant or bridge-to-recovery is on the rise. Thousands of patients with left-sided heart failure are readily benefitting from these life-saving technologies, and left ventricular failure often leads to severe right ventricular dysfunction or failure. Right ventricular failure (RVF) has a high rate of mortality caused by the risk of multisystem organ failure and prolonged hospitalization for patients after treatment...
May 10, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29756719/-pulmonary-hypertension-associated-with-the-human-immunodeficiency-virus-in-children-treatment-with-sildenafil-a-case-report
#6
Carolina Stepffer, Evelyn Gaynor, Mariela López, Norma E González, Micaela Arri, Ana Ms De Dios
Pulmonary hypertension associated with human immunodeficiency virus infection is an extremely rare disease in pediatrics; it requires a high clinical suspicion to reach a diagnosis. Its appearance poses an unfavorable prognostic, but early diagnosis and specific treatment can improve outcomes. We report the clinical case of a fifteen-year-old patient diagnosed with human immunodeficiency virus infection of vertical transmission, without antiretroviral treatment, with cough and progressive exertional dyspnea, associated with signs of right heart failure in which severe pulmonary hypertension was diagnosed...
June 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29754190/allergic-and-immunologic-perspectives-of-inflammatory-bowel-disease
#7
REVIEW
Kofi Clarke, Jayakrishna Chintanaboina
Inflammatory bowel disease (IBD) is a chronic immune-mediated inflammatory condition primarily involving the gastrointestinal tract. It includes Crohn's disease (CD), ulcerative colitis (UC), and a less common phenotype-indeterminate colitis. It is thought to result from a complex interplay of environmental, microbial, and host factors including genetic factors, although the exact mechanism is not known. Dietary factors have been shown to play a role in the pathogenesis of IBD and can potentially alter the intestinal microbiota as well as disrupt the immune function in the gut...
May 12, 2018: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29753543/increased-fracture-risk-with-furosemide-use-in-children-with-congenital-heart-disease
#8
Ji Haeng Heo, Karen L Rascati, Keila N Lopez, Brady S Moffett
OBJECTIVES: To determine the association of furosemide therapy with the incidence of bone fractures in children with congenital heart disease. STUDY DESIGN: We conducted a retrospective cohort study with data extracted from the 2008-2014 Texas Medicaid databases. Pediatric patients aged <12 years diagnosed with congenital heart disease, cardiomyopathy, or heart failure were included. Patients taking furosemide were categorized into a furosemide-adherent group (medication possession ratio of ≥70%), and a furosemide-nonadherent group (medication possession ratio of <70%)...
May 9, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29749291/antithrombotic-therapy-in-pediatric-ventricular-assist-devices-multicenter-survey-of-the-european-excor-pediatric-investigator-group
#9
Oliver Miera, Katharina L Schmitt, Hakan Akintuerk, Angele Boet, Robert Cesnjevar, Teresa Chila, Thilo Fleck, Ranny Goldwasser, Luis G Guereta, Beatrice Heineking, Juergen Hoerer, Alexander Horke, Tain Y Hsia, Michael Huebler, Andrzej Kansy, Ann Karimova, Bohdan Maruszewski, Constancio Medrano, Szymon Pawlak, Zdenka Reinhardt, Birgitta Romlin, Eugen Sandica, Florian Schmidt, René Schramm, Martin Schweiger, Joanna Śliwka, Brigitte Stiller, Josef Thul, Antonio Amodeo
OBJECTIVES: Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members. METHODS: We sent a questionnaire assessing seven aspects of antithrombotic therapy to 18 European hospitals using the EXCOR device for children...
May 1, 2018: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/29746377/risk-factors-for-severe-community-acquired-pneumonia-among-children-hospitalized-with-cap-younger-than-5-years-of-age
#10
Wei Shan, Ting Shi, Kaile Chen, Jian Xue, Yin Wang, Jia Yu, Genming Zhao, Jianmei Tian, Tao Zhang
BACKGROUND: CAP causes great morbidity and mortality as well as enormous economic burden worldwide. This study intended to describe the clinical characteristics of CAP and explore the risk factors of severe CAP among children in downtown Suzhou, China. METHODS: This was a retrospective study of childhood hospitalizations in Soochow University Affiliated Children's Hospital (SCH) from January 1, 2010 to December 31, 2014. Children who were residents of downtown Suzhou, 29 days to <5 years of age, with discharge diagnosis codes (ICD-10) J09 to J18 and J20 to J22 were included...
May 9, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29744657/transcatheter-intervention-of-coronary-obstructions-in-infants-children-and-young-adults
#11
Ryan Callahan, James E Lock, Pinak B Shah, Audrey C Marshall
This study assesses the characteristics of coronary obstructions that underwent transcatheter intervention in a pediatric catheterization laboratory, the procedural details, and patient outcomes. Acute cardiac failure due to coronary obstructions in children is rare. The role of catheter based intervention is largely unreported. Single center retrospective review between January 2000 and December 2016. Thirty-three patients (median age 2y/o [0-38], weighing 9.6 kg [2.2-91]) underwent 39 transcatheter interventions on 39 lesions, mainly left main coronary (16/39; 39%) and right coronary (9/39; 23%) arteries...
May 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29731013/hypertension-canada-s-2018-guidelines-for-diagnosis-risk-assessment-prevention-and-treatment-of-hypertension-in-adults-and-children
#12
Kara A Nerenberg, Kelly B Zarnke, Alexander A Leung, Kaberi Dasgupta, Sonia Butalia, Kerry McBrien, Kevin C Harris, Meranda Nakhla, Lyne Cloutier, Mark Gelfer, Maxime Lamarre-Cliche, Alain Milot, Peter Bolli, Guy Tremblay, Donna McLean, Raj S Padwal, Karen C Tran, Steven Grover, Simon W Rabkin, Gordon W Moe, Jonathan G Howlett, Patrice Lindsay, Michael D Hill, Mike Sharma, Thalia Field, Theodore H Wein, Ashkan Shoamanesh, George K Dresser, Pavel Hamet, Robert J Herman, Ellen Burgess, Steven E Gryn, Jean C Grégoire, Richard Lewanczuk, Luc Poirier, Tavis S Campbell, Ross D Feldman, Kim L Lavoie, Ross T Tsuyuki, George Honos, Ally P H Prebtani, Gregory Kline, Ernesto L Schiffrin, Andrew Don-Wauchope, Sheldon W Tobe, Richard E Gilbert, Lawrence A Leiter, Charlotte Jones, Vincent Woo, Robert A Hegele, Peter Selby, Andrew Pipe, Philip A McFarlane, Paul Oh, Milan Gupta, Simon L Bacon, Janusz Kaczorowski, Luc Trudeau, Norman R C Campbell, Swapnil Hiremath, Michael Roerecke, Joanne Arcand, Marcel Ruzicka, G V Ramesh Prasad, Michel Vallée, Cedric Edwards, Praveena Sivapalan, S Brian Penner, Anne Fournier, Geneviève Benoit, Janusz Feber, Janis Dionne, Laura A Magee, Alexander G Logan, Anne-Marie Côté, Evelyne Rey, Tabassum Firoz, Laura M Kuyper, Jonathan Y Gabor, Raymond R Townsend, Doreen M Rabi, Stella S Daskalopoulou
Hypertension Canada provides annually updated, evidence-based guidelines for the diagnosis, assessment, prevention, and treatment of hypertension in adults and children. This year, the adult and pediatric guidelines are combined in one document. The new 2018 pregnancy-specific hypertension guidelines are published separately. For 2018, 5 new guidelines are introduced, and 1 existing guideline on the blood pressure thresholds and targets in the setting of thrombolysis for acute ischemic stroke is revised. The use of validated wrist devices for the estimation of blood pressure in individuals with large arm circumference is now included...
May 2018: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/29717916/risk-factors-associated-with-the-development-of-acquired-airway-disease-after-congenital-heart-surgery-a-retrospective-cohort-study
#13
Andrew J Matisoff, Pranathi Ari, David Zurakowski, Alexandra G Espinel, Nina Deutsch, Brian K Reilly
OBJECTIVE: In this single-center, retrospective review, we sought to determine the risk factors associated with the development of severe acquired airway disease (AAD; vocal cord paralysis [VCP] or subglottic stenosis [SGS]) in pediatric patients who had undergone surgery for congenital heart disease (CHD) with cardiopulmonary bypass. All patients who required surgical treatment for CHD using cardiopulmonary bypass at our institution between 2010 and 2015 were reviewed. We defined severe AAD as either clinically significant VCP, SGS, or both, requiring consultation with the otolaryngology (ENT) service for evaluation...
April 1, 2018: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29710196/long-term-outcomes-of-pediatric-onset-hypertrophic-cardiomyopathy-and-age-specific-risk-factors-for-lethal-arrhythmic-events
#14
Niccolò Maurizi, Silvia Passantino, Gaia Spaziani, Francesca Girolami, Anna Arretini, Mattia Targetti, Iva Pollini, Alessia Tomberli, Silvia Pradella, Giovanni Battista Calabri, Veronica Vinattieri, Bruno Bertaccini, Ornella Leone, Luciano De Simone, Claudio Rapezzi, Niccolò Marchionni, Franco Cecchi, Silvia Favilli, Iacopo Olivotto
Importance: Predictors of lethal arrhythmic events (LAEs) after a pediatric diagnosis of hypertrophic cardiomyopathy (HCM) are unresolved. Existing algorithms for risk stratification are limited to patients older than 16 years because of a lack of data on younger individuals. Objective: To describe the long-term outcome of pediatric-onset HCM and identify age-specific arrhythmic risk factors. Design, Setting, and Participants: This study assessed patients with pediatric-onset hypertrophic cardiomyopathy diagnosed from 1974 to 2016 in 2 national referral centers for cardiomyopathies in Florence, Italy...
April 18, 2018: JAMA Cardiology
https://www.readbyqxmd.com/read/29703578/early-in-vivo-experience-with-the-pediatric-continuous-flow-total-artificial-heart
#15
Jamshid H Karimov, David J Horvath, Nicole Byram, Gengo Sunagawa, Barry D Kuban, Shengqiang Gao, Raymond Dessoffy, Kiyotaka Fukamachi
BACKGROUND: Heart transplantation in infants and children is an accepted therapy for end-stage heart failure, but donor organ availability is low and always uncertain. Mechanical circulatory support is another standard option, but there is a lack of intracorporeal devices due to size and functional range. The purpose of this study was to evaluate the in vivo performance of our initial prototype of a pediatric continuous-flow total artificial heart (P-CFTAH), comprising a dual pump with one motor and one rotating assembly, supported by a hydrodynamic bearing...
March 30, 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29690950/safety-of-tracheal-intubation-in-the-presence-of-cardiac-disease-in-paediatric-icus
#16
Eleanor A Gradidge, Adnan Bakar, David Tellez, Michael Ruppe, Sarah Tallent, Geoffrey Bird, Natasha Lavin, Anthony Lee, Vinay Nadkarni, Michelle Adu-Darko, Jesse Bain, Katherine Biagas, Aline Branca, Ryan K Breuer, Calvin Brown, Kris Bysani, Guillaume Emeriaud, Sandeep Gangadharan, John S Giuliano, Joy D Howell, Conrad Krawiec, Jan Hau Lee, Simon Li, Keith Meyer, Michael Miksa, Natalie Napolitano, Sholeen Nett, Gabrielle Nuthall, Alberto Orioles, Erin B Owen, Margaret M Parker, Simon Parsons, Lee A Polikoff, Kyle Rehder, Osamu Saito, Ron C Sanders, Asha Shenoi, Dennis W Simon, Peter W Skippen, Keiko Tarquinio, Anne Thompson, Iris Toedt-Pingel, Karen Walson, Akira Nishisaki
IntroductionChildren with CHD and acquired heart disease have unique, high-risk physiology. They may have a higher risk of adverse tracheal-intubation-associated events, as compared with children with non-cardiac disease.Materials and methodsWe sought to evaluate the occurrence of adverse tracheal-intubation-associated events in children with cardiac disease compared to children with non-cardiac disease. A retrospective analysis of tracheal intubations from 38 international paediatric ICUs was performed using the National Emergency Airway Registry for Children (NEAR4KIDS) quality improvement registry...
April 25, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29685473/acute-pediatric-colchicine-toxicity-is-associated-with-marked-bradydysrhythmias
#17
Lamya Mubayed, Brie-Ann A Muller, Jessica L Jacobson, Howard A Hast, Hoang H Nguyen
BACKGROUND: Colchicine ingestion is rare but highly lethal. Patients usually die of multiorgan failure and cardiogenic shock. Colchicine is not only associated with depressed myocardial function but also with fatal heart rhythm disturbances, such as complete heart block, ventricular tachycardia, and asystole. While histologic changes of myocytes are well known, the mechanism by which colchicine affects cardiac impulse generation and conduction is not fully understood. CASE REPORT: We present a case of colchicine ingestion with sinus bradycardia, marked sinus arrhythmia, and first- and second-degree heart block...
April 20, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29668027/heart-transplant-in-children-what-a-primary-care-provider-needs-to-know
#18
Swati Sehgal, Emily Shea, Lauren Kelm, Deepak Kamat
Heart transplantation is offered to children with heart failure that is not amenable to medical or surgical therapy. Indications for heart transplant include unrepairable congenital heart disease, failed palliation of congenital heart disease, and cardiomyopathies. There has been tremendous progress in this field since the first heart transplant was performed in 1967. Each year, approximately 500 pediatric heart transplants take place worldwide. Pediatric heart transplant survivors are living longer with their initial transplant...
April 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29666009/in-vitro-validation-of-a-self-driving-aortic-turbine-venous-assist-device-for-fontan-patients
#19
Kerem Pekkan, Ibrahim Basar Aka, Ece Tutsak, Erhan Ermek, Haldun Balim, Ismail Lazoglu, Riza Turkoz
BACKGROUND: Palliative repair of single ventricle defects involve a series of open-heart surgeries where a single-ventricle (Fontan) circulation is established. As the patient ages, this paradoxical circulation gradually fails, because of its high venous pressure levels. Reversal of the Fontan paradox requires an extra subpulmonic energy that can be provided through mechanical assist devices. The objective of this study was to evaluate the hemodynamic performance of a totally implantable integrated aortic-turbine venous-assist (iATVA) system, which does not need an external drive power and maintains low venous pressure chronically, for the Fontan circulation...
March 11, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29620642/in-hospital-mortality-and-morbidity-of-pediatric-scoliosis-surgery-in-japan-analysis-using-a-national-inpatient-database
#20
Yuki Taniguchi, Takeshi Oichi, Junichi Ohya, Hirotaka Chikuda, Yasushi Oshima, Yoshitaka Matsubayashi, Hiroki Matsui, Kiyohide Fushimi, Sakae Tanaka, Hideo Yasunaga
Several previous reports have elucidated the mortality and incidence of complications after pediatric scoliosis surgery using nationwide databases. However, all of these studies were conducted in North America. Hence, this study aimed to identify the incidence and risk factors for in-hospital mortality and morbidity in pediatric scoliosis surgery, utilizing the Diagnosis Procedure Combination database, a national inpatient database in Japan.We retrospectively extracted data for patients aged less than 19 years who were admitted between 01 June 2010 and 31 March 2013 and underwent scoliosis surgery with fusion...
April 2018: Medicine (Baltimore)
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